Rare disease
CASE REPORT
A 16-year-old boy with emphysematous gastritis and oesophageal candidiasis Daniel Yusef, Ariane Waran, Ekaterini Vamvakiti Department of Paediatrics, Western Sussex Hospitals Trust, Worthing, W Sussex, UK Correspondence to Dr Daniel Yusef, [email protected] Accepted 18 August 2014
SUMMARY Emphysematous gastritis is a rare and frequently fatal condition caused by invasion of gas-forming bacteria into the gastric wall. There have only been a handful of reported cases in the paediatric population, and none of these have evidence of candidal infection or mucormycosis. Patients typically present with abdominal pain, vomiting, malaena and haematemesis. Risk factors for emphysematous gastritis are those that interfere with the natural barriers to infection in the stomach. Diagnosis is made on the basis of typical appearances on abdominal CT. Treatment is generally conservative with surgery reserved for failed medical management or later complications. Antimicrobial cover should be broad with a low threshold for antifungals. It is important to look for predisposing factors for this condition, perhaps including an assessment of the patient’s immunocompetency. We present a 16-year-old boy with global developmental delay who presented with this condition associated with candidal infection.
CASE PRESENTATION Our patient was a 16-year-old boy with a history of global developmental delay, spastic quadriplaegia and a ventriculoperitoneal shunt for hydrocephalus in situ. A gastrostomy had been inserted several years previously due to gastro-oesophageal reflux and feeding difficulties. The patient had had a right orchidectomy 7 days prior to presentation for an infarcted testis. He had received 5 days of oral co-amoxiclav following this. His only other medication was a regular laxative for constipation. The patient presented to our department with a short history of haematemesis, melaena and abdominal pain. The HR was 96, he was warm and well perfused, his respiratory rate was 20, he was afebrile and had oxygen saturations of 98%. On examination, he was found to be distressed and pale with a distended, generally tender abdomen but with no signs of peritonism. The site of his previous surgical incision looked healthy and not infected. Over the course of the next few hours the percutaneous endoscopic gastrostomy tube drained ‘coffeeground’ fluid, he developed signs of peritonism and he became increasingly tachycardic (HR=155).
same day. Renal function tests revealed urea of 14.1 mmol/L, but other parameters were within normal range. Liver function tests were normal including transaminases. A CT of the abdomen and pelvis was performed, which showed the walls of the distal oesophagus and stomach were thickened with diffuse areas of intramural gas. An oesophagogastroduodenoscopy (OGD) was undertaken to further evaluate these findings. This showed severe oesophageal candidiasis and inflammation around the gastro-oesophageal junction. There was no evidence of perforation. Biopsies were not taken for fear of causing iatrogenic perforation. Blood cultures and swabs taken from the orchidectomy site were sterile. These were taken before antibiotics or antifungals. Immunodeficiency screening tests: total protein 73 g/L (60–80), albumin 44 g/L (35–50), IgG 6.53 g/L (6–16), IgM 1.35 g/L (0.5–1.9), IgA 3.83 g/L (0.8–2.8), IgG1 2.96 g/L (3.2–10.2), IgG2 3 g/L (1.2–6.6), IgG3 1.25 g/L (0.2–1.9), IgG4 0.77 g/L (0.1–1.3), CD4 35% (25–48), CD4 total 1900×106/L (400–2100), CD8 50% (9–35), CD8 total 2670×106/L (200–1200), CD4:CD8 ratio 0.7 (0.9–3.4), response to vaccines Hib/DPT normal.
DIFFERENTIAL DIAGNOSIS ▸ Emphysematous gastritis ▸ Gastric emphysema
OUTCOME AND FOLLOW-UP Our patient was managed with intravenous fluid boluses, omeprazole and a 15mL/kg packed red cell transfusion. Following this he remained cardiovascularly stable. He was initially started on teicoplanin, gentamicin and tazocin (tazobactam and piperacillin). Following the results of the OGD he was also started on fluconazole intravenously. The antibiotics and antifungals were continued for a total of 14 days. Feeds were initially withheld but were gradually restarted on day 2 of the illness and he was fully enterally fed by day 7 of the admission. Blood parameters gradually returned to normal, and there was no further evidence of enteral bleeding.
INVESTIGATIONS To cite: Yusef D, Waran A, Vamvakiti E. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014203755
A laboratory workup showed a haemoglobin of 8.4 g/dL, white cell count 27.2×109/L, with a neutrophil count 19.2×109/L. The international normalized ratio was 1.2 and activated partial thromboplastin time was 1.1. The C reactive protein was 56 but rose to 397 by the end of the
DISCUSSION Emphysematous gastritis is a rare and frequently fatal condition caused by invasion of gas-forming bacteria into the gastric wall. There have only been a handful of reported cases in the paediatric population, and none of these have evidence of candidal
Yusef D, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-203755
1
Rare disease infection or mucormycosis.1 2 The first description of emphysematous gastritis was in 1889.3 The condition has a poor prognosis with mortality of 55–61%.4 5 Patients with emphysematous gastritis will present with acute abdominal pain often accompanied by nausea, vomiting and diarrhoea as well as haematemesis and melaena.6 Physical examination reveals a toxic appearance, an acute ‘surgical’ abdomen with distension, guarding and absent or decreased bowel sounds. Laboratory investigations typically reveal a leucocytosis and a metabolic acidosis. Liver enzymes and alkaline phosphatases may also be raised.7 Diagnosis is typically made on the basis of radiological imaging. The most useful medium is CT, which typically shows gastric wall thickening and intramural gas.6 7 The gas in the wall of the stomach typically collects around the fundus and the greater curvature and forms irregular bubbles6 (figures 1 and 2). Endoscopy can be helpful and may show bubbles beneath the gastric mucosa or features of necrosis.7 Emphysematous gastritis should be distinguished from gastric emphysema. Gastric emphysema is a much more benign condition that in itself normally requires no treatment. It is due to mechanical redistribution of air from the lumen of the stomach into the gastric wall. Typically this occurs following interventions such as nasogastric tube insertion or instrumentation. It can also occur in situations where there is raised intraluminal pressure such as distal obstruction or forceful vomiting.7
Figure 2 the abdominal CT showing the typical appearances of emphysematous gastritis. There is prominent gastric intramural gas seen. A gastrostomy tube is also in situ. There have been a number of predisposing factors for emphysematous gastritis identified in the literature. Risk factors for emphysematous gastritis are those that interfere with the natural barriers to infection in the stomach. Factors that have been identified include: ingestion of corrosives, alcohol abuse, recent abdominal surgery and any condition affecting the immunocompetency of the host.6 7 In our case no predisposing factors were identified. The causative organisms can be Gram positive, Gram negative, anaerobic bacteria or sometimes fungal.6 7 Although rare, fungal mucormycosis in the context of emphysematous gastritis has been described in the literature, although never in children. In these patients Candida is the most common culprit.6 Since Candida is not gas forming it is likely that in these cases there is concomitant bacterial invasion of the stomach wall. In our case, we cannot prove invasive candidal disease but we presumed it on the basis of the macroscopic endoscopic findings. Current consensus is conservative management with broad-spectrum antibiotics and antifungals along with aggressive fluid management; surgery is reserved for those cases resistant to medical treatment.6 7 In the longer term there is the risk of gastric strictures, which may require surgical intervention.
Learning points ▸ Diagnosis is made on the basis of a typical history and appearances on CT. ▸ Treatment of emphysematous gastritis is normally supportive with surgery reserved for failed medical management or later complications. ▸ Antimicrobial cover should be broad with a low threshold for antifungals. ▸ The cause of this condition should be considered and may include work up for an immunodeficiency.
Figure 1 The abdominal CT showing the typical appearances of emphysematous gastritis. There is prominent gastric intramural gas seen. A gastrostomy tube is also in situ. 2
Contributors All three authors have made substantial contributions to the acquisition, analysis and interpretation of data for the work. In addition, they were involved in drafting the work and revising it critically. DY is the guarantor. Competing interests None. Yusef D, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-203755
Rare disease Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1
2
Eisenhut M, Hughes D, Ashworth M. Fatal emphysematous gastritis in a 2-year-old child with chronic renal failure. Pediatr Dev Pathol 2004;7:414–16. Rowen M, Myers M, Williamson RA. Emphysematous gastritis in a leukaemic child. Med Pediatr Oncol 1976;2:433–7.
3 4 5 6 7
Fraenkel E. A case of emphysematous gastritis probably of mucomycotic origin [in German]. Virchows Arch A 1889;118:526–35. Haung CT, Liao WY. Emphysematous gastritis: a deadly infectious disease. Scand J Infect Dis 2009;41:317–19. Moosvi AR, Saravolatz LD, Wong DH, et al. Emphysematous gastritis: case report and review. Rev Infect Dis 1990;12:848–55. Jung JH, Choi HJ, Yoo J, et al. Emphysematous gastritis associated with invasive gastric mucormycosis: a case report. J Korean Med Sci 2007;22:923–7. Yalamachili M, Cady W. Emphysematous gastritis in a hemodialysis patient. Sout Med J 2003:96:84–8.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Yusef D, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-203755
3
CASE REPORT
A 16-year-old boy with emphysematous gastritis and oesophageal candidiasis Daniel Yusef, Ariane Waran, Ekaterini Vamvakiti Department of Paediatrics, Western Sussex Hospitals Trust, Worthing, W Sussex, UK Correspondence to Dr Daniel Yusef, [email protected] Accepted 18 August 2014
SUMMARY Emphysematous gastritis is a rare and frequently fatal condition caused by invasion of gas-forming bacteria into the gastric wall. There have only been a handful of reported cases in the paediatric population, and none of these have evidence of candidal infection or mucormycosis. Patients typically present with abdominal pain, vomiting, malaena and haematemesis. Risk factors for emphysematous gastritis are those that interfere with the natural barriers to infection in the stomach. Diagnosis is made on the basis of typical appearances on abdominal CT. Treatment is generally conservative with surgery reserved for failed medical management or later complications. Antimicrobial cover should be broad with a low threshold for antifungals. It is important to look for predisposing factors for this condition, perhaps including an assessment of the patient’s immunocompetency. We present a 16-year-old boy with global developmental delay who presented with this condition associated with candidal infection.
CASE PRESENTATION Our patient was a 16-year-old boy with a history of global developmental delay, spastic quadriplaegia and a ventriculoperitoneal shunt for hydrocephalus in situ. A gastrostomy had been inserted several years previously due to gastro-oesophageal reflux and feeding difficulties. The patient had had a right orchidectomy 7 days prior to presentation for an infarcted testis. He had received 5 days of oral co-amoxiclav following this. His only other medication was a regular laxative for constipation. The patient presented to our department with a short history of haematemesis, melaena and abdominal pain. The HR was 96, he was warm and well perfused, his respiratory rate was 20, he was afebrile and had oxygen saturations of 98%. On examination, he was found to be distressed and pale with a distended, generally tender abdomen but with no signs of peritonism. The site of his previous surgical incision looked healthy and not infected. Over the course of the next few hours the percutaneous endoscopic gastrostomy tube drained ‘coffeeground’ fluid, he developed signs of peritonism and he became increasingly tachycardic (HR=155).
same day. Renal function tests revealed urea of 14.1 mmol/L, but other parameters were within normal range. Liver function tests were normal including transaminases. A CT of the abdomen and pelvis was performed, which showed the walls of the distal oesophagus and stomach were thickened with diffuse areas of intramural gas. An oesophagogastroduodenoscopy (OGD) was undertaken to further evaluate these findings. This showed severe oesophageal candidiasis and inflammation around the gastro-oesophageal junction. There was no evidence of perforation. Biopsies were not taken for fear of causing iatrogenic perforation. Blood cultures and swabs taken from the orchidectomy site were sterile. These were taken before antibiotics or antifungals. Immunodeficiency screening tests: total protein 73 g/L (60–80), albumin 44 g/L (35–50), IgG 6.53 g/L (6–16), IgM 1.35 g/L (0.5–1.9), IgA 3.83 g/L (0.8–2.8), IgG1 2.96 g/L (3.2–10.2), IgG2 3 g/L (1.2–6.6), IgG3 1.25 g/L (0.2–1.9), IgG4 0.77 g/L (0.1–1.3), CD4 35% (25–48), CD4 total 1900×106/L (400–2100), CD8 50% (9–35), CD8 total 2670×106/L (200–1200), CD4:CD8 ratio 0.7 (0.9–3.4), response to vaccines Hib/DPT normal.
DIFFERENTIAL DIAGNOSIS ▸ Emphysematous gastritis ▸ Gastric emphysema
OUTCOME AND FOLLOW-UP Our patient was managed with intravenous fluid boluses, omeprazole and a 15mL/kg packed red cell transfusion. Following this he remained cardiovascularly stable. He was initially started on teicoplanin, gentamicin and tazocin (tazobactam and piperacillin). Following the results of the OGD he was also started on fluconazole intravenously. The antibiotics and antifungals were continued for a total of 14 days. Feeds were initially withheld but were gradually restarted on day 2 of the illness and he was fully enterally fed by day 7 of the admission. Blood parameters gradually returned to normal, and there was no further evidence of enteral bleeding.
INVESTIGATIONS To cite: Yusef D, Waran A, Vamvakiti E. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014203755
A laboratory workup showed a haemoglobin of 8.4 g/dL, white cell count 27.2×109/L, with a neutrophil count 19.2×109/L. The international normalized ratio was 1.2 and activated partial thromboplastin time was 1.1. The C reactive protein was 56 but rose to 397 by the end of the
DISCUSSION Emphysematous gastritis is a rare and frequently fatal condition caused by invasion of gas-forming bacteria into the gastric wall. There have only been a handful of reported cases in the paediatric population, and none of these have evidence of candidal
Yusef D, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-203755
1
Rare disease infection or mucormycosis.1 2 The first description of emphysematous gastritis was in 1889.3 The condition has a poor prognosis with mortality of 55–61%.4 5 Patients with emphysematous gastritis will present with acute abdominal pain often accompanied by nausea, vomiting and diarrhoea as well as haematemesis and melaena.6 Physical examination reveals a toxic appearance, an acute ‘surgical’ abdomen with distension, guarding and absent or decreased bowel sounds. Laboratory investigations typically reveal a leucocytosis and a metabolic acidosis. Liver enzymes and alkaline phosphatases may also be raised.7 Diagnosis is typically made on the basis of radiological imaging. The most useful medium is CT, which typically shows gastric wall thickening and intramural gas.6 7 The gas in the wall of the stomach typically collects around the fundus and the greater curvature and forms irregular bubbles6 (figures 1 and 2). Endoscopy can be helpful and may show bubbles beneath the gastric mucosa or features of necrosis.7 Emphysematous gastritis should be distinguished from gastric emphysema. Gastric emphysema is a much more benign condition that in itself normally requires no treatment. It is due to mechanical redistribution of air from the lumen of the stomach into the gastric wall. Typically this occurs following interventions such as nasogastric tube insertion or instrumentation. It can also occur in situations where there is raised intraluminal pressure such as distal obstruction or forceful vomiting.7
Figure 2 the abdominal CT showing the typical appearances of emphysematous gastritis. There is prominent gastric intramural gas seen. A gastrostomy tube is also in situ. There have been a number of predisposing factors for emphysematous gastritis identified in the literature. Risk factors for emphysematous gastritis are those that interfere with the natural barriers to infection in the stomach. Factors that have been identified include: ingestion of corrosives, alcohol abuse, recent abdominal surgery and any condition affecting the immunocompetency of the host.6 7 In our case no predisposing factors were identified. The causative organisms can be Gram positive, Gram negative, anaerobic bacteria or sometimes fungal.6 7 Although rare, fungal mucormycosis in the context of emphysematous gastritis has been described in the literature, although never in children. In these patients Candida is the most common culprit.6 Since Candida is not gas forming it is likely that in these cases there is concomitant bacterial invasion of the stomach wall. In our case, we cannot prove invasive candidal disease but we presumed it on the basis of the macroscopic endoscopic findings. Current consensus is conservative management with broad-spectrum antibiotics and antifungals along with aggressive fluid management; surgery is reserved for those cases resistant to medical treatment.6 7 In the longer term there is the risk of gastric strictures, which may require surgical intervention.
Learning points ▸ Diagnosis is made on the basis of a typical history and appearances on CT. ▸ Treatment of emphysematous gastritis is normally supportive with surgery reserved for failed medical management or later complications. ▸ Antimicrobial cover should be broad with a low threshold for antifungals. ▸ The cause of this condition should be considered and may include work up for an immunodeficiency.
Figure 1 The abdominal CT showing the typical appearances of emphysematous gastritis. There is prominent gastric intramural gas seen. A gastrostomy tube is also in situ. 2
Contributors All three authors have made substantial contributions to the acquisition, analysis and interpretation of data for the work. In addition, they were involved in drafting the work and revising it critically. DY is the guarantor. Competing interests None. Yusef D, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-203755
Rare disease Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1
2
Eisenhut M, Hughes D, Ashworth M. Fatal emphysematous gastritis in a 2-year-old child with chronic renal failure. Pediatr Dev Pathol 2004;7:414–16. Rowen M, Myers M, Williamson RA. Emphysematous gastritis in a leukaemic child. Med Pediatr Oncol 1976;2:433–7.
3 4 5 6 7
Fraenkel E. A case of emphysematous gastritis probably of mucomycotic origin [in German]. Virchows Arch A 1889;118:526–35. Haung CT, Liao WY. Emphysematous gastritis: a deadly infectious disease. Scand J Infect Dis 2009;41:317–19. Moosvi AR, Saravolatz LD, Wong DH, et al. Emphysematous gastritis: case report and review. Rev Infect Dis 1990;12:848–55. Jung JH, Choi HJ, Yoo J, et al. Emphysematous gastritis associated with invasive gastric mucormycosis: a case report. J Korean Med Sci 2007;22:923–7. Yalamachili M, Cady W. Emphysematous gastritis in a hemodialysis patient. Sout Med J 2003:96:84–8.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Yusef D, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-203755
3
![[A rare and fulminant form of gastritis: emphysematous gastritis].](/assets/img/hold.png)
